J Neurol Neurosurg Psychiatry. 2008 Apr;79(4):467-70. Epub 2007 Oct
2. Links
Aceruloplasminaemia with progressive atrophy without brain iron
overload: treatment with oral chelation.
Skidmore FM, Drago V, Foster P, Schmalfuss IM, Heilman KM, Streiff RR.
University of Florida School of Medicine, North Florida/South Georgia
VA Medical Center, McKnight Brain Institute at UF, 100 S. Newell
Drive, Room L3-100, PO Box 100236, Gainesville, FL 32610-0236, USA.
frank.skidmore@neurology.ufl.edu

BACKGROUND:
Hereditary aceruloplasminaemia is a disorder of iron metabolism that
is characterised by iron accumulation in the brain and other visceral
organs. In previously reported cases, individuals with the disorder
were noted to have evidence of iron accumulation in the brain. Oral
chelating agents have not been used in neurological diseases of iron
metabolism.
METHODS:
A 54-year-old woman who presented with ataxia, lower extremity
spasticity and chorea was evaluated for evidence of the source of
neurological dysfunction. RESULTS: Blood studies revealed no
detectable ceruloplasmin. Marked iron overload was defined by a liver
biopsy, which showed a variegated pattern consistent with a primary
cause of iron overload. Review of MRI scans showed progressive brain
atrophy without visible iron accumulation occurring over a 5-year
period. The history suggested that neurodegeneration was coincident
with aggressive oral iron replacement. Oral chelation improved many
symptoms. CONCLUSIONS:
Our findings in this patient suggest that disorders of iron transport
such as aceruloplasminaemia can be a cause of neurological symptoms
such as chorea and cognitive decline, as well as progressive
neurodegeneration in the absence of visible iron on MRI scans. We
found that oral iron chelation was effective at improving symptoms.

PMID: 17911185

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